Epidemiology and Outcome of Invasive Fungal Diseases in Patients With Chronic Granulomatous Disease
- 1 January 2011
- journal article
- research article
- Published by Ovid Technologies (Wolters Kluwer Health) in The Pediatric Infectious Disease Journal
- Vol. 30 (1), 57-62
- https://doi.org/10.1097/inf.0b013e3181f13b23
Abstract
Chronic granulomatous disease (CGD) is a rare inherited phagocytic disorder resulting in an increased susceptibility to infections including invasive fungal diseases (IFDs) and inflammatory complications. This study is aimed at assessing the incidence, prevalence, and outcome of IFDs among CGD patients followed in France. CGD patients were identified through the French national registry for primary immunodeficiencies (PID) held by the French national reference Centre of PID (Centre de Référence Déficits Immunitaires Héréditaires), which comprises a total of 3083 patients including 155 with CGD followed between 1976 and 2008. A questionnaire was filled out for each episode of IFD. Information retrieved included a description of the IFD using the 2008 European Organization for Research and Treatment of Cancer/Mycoses Study Group IFD definition criteria. Of CGD patients, 42.6% (66/155) developed at least 1 episode of IFD. Overall incidence of IFD was 0.040/patient-years (1862 patient-years of total follow-up). IFD incidence was found to be significant while receiving itraconazole prophylaxis compared with no prophylaxis (0.027 vs. 0.053 IFD/patient-years; P < 0.01). Median age at IFD diagnosis was 6.5 years (3.3-11.3). The most common fungal genus was Aspergillus sp. accounting for 40% of all IFDs. Of the IFDs, 42.5% were proven, 30.0% probable, and 27.5% possible. Of all IFD episodes, 52.5% were treated by antifungal monotherapy, mostly by amphotericin B. Survival was reduced in IFD patients compared with those without it (log-rank 0.04). IFDs are a frequent and life-threatening complication in CGD patients. Itraconazole significantly reduces its incidence and should be recommended in absence of better alternatives.Keywords
This publication has 31 references indexed in Scilit:
- A new genetic subgroup of chronic granulomatous disease with autosomal recessive mutations in p40phox and selective defects in neutrophil NADPH oxidase activityBlood, 2009
- Invasive aspergillosis in chronic granulomatous diseaseMedical Mycology, 2009
- Special Article: Chronic granulomatous disease in the United Kingdom and Ireland: a comprehensive national patient-based registryClinical and Experimental Immunology, 2008
- Clinical features, long-term follow-up and outcome of a large cohort of patients with Chronic Granulomatous Disease: An Italian multicenter studyClinical Immunology, 2008
- Fungal infections in primary immunodeficienciesEuropean Journal of Pediatrics, 2007
- Successful Low Toxicity Hematopoietic Stem Cell Transplantation for High-Risk Adult Chronic Granulomatous Disease PatientsTransplantation, 2005
- Invasive aspergillosis in primary immunodeficienciesMedical Mycology, 2005
- Treatment of chronic granulomatous disease with myeloablative conditioning and an unmodified hemopoietic allograft: a survey of the European experience, 1985-2000Blood, 2002
- Chronic Granulomatous Disease: Report on a National Registry of 368 PatientsMedicine, 2000
- Chronic granulomatous disease in Japan: Incidence and natural historyPediatrics International, 1999